Haematologica (HAEMATOL-HEMATOL J)

Publications in this journal

• Article: Sildenafil therapy in thalassemia patients with doppler-defined risk for pulmonary hypertension

  Claudia R. Morris, Hae-Young Kim, John Wood, John B. Porter, Elizabeth S. Klings, Felicia L. Trachtenberg, Nancy Sweeters, Nancy F Olivieri, Janet L Kwiatkowski, Lisa Virzi, Sylvia T. Singer, Ali Taher, Ellis J Neufeld, Alexis A. Thompson, Vandana Sachdev, Sandra Larkin, Jung H. Suh, Frans A. Kuypers, Elliott P. Vichinsky
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  ABSTRACT: Pulmonary hypertension is a common but often overlooked complication associated with thalassemia syndromes. There is limited data on the safety and efficacy of selective pulmonary vasodilators in this at-risk population. We therefore designed a 12-week open-label phase 1/2 pilot-scale proof-of-principle trial of sildenafil therapy in 10 patients with beta-thalassemia and increased risk for pulmonary hypertension based upon an elevated tricuspid regurgitant jet velocity >2.5 m/s on Doppler-echocardiography. Variables measured include Doppler-echocardiography, 6-minute walk distance, Borg Dyspnea Score, New York Heart Association functional class, pulmonary function testing, and laboratory analyses compared at baseline and after 12 weeks of sildenafil. Treatment with sildenafil resulted in a significant decrease in tricuspid regurgitant jet velocity by 13.3% (3.0+/-0.7 vs. 2.6+/-0.5m/s, p=0.04), improved left ventricular end systolic/diastolic volume, and a trend towards a improved New York Heart Association functional class. No significant change in six-minute walk distance was noted. Sildenafil was well tolerated, although minor anticipated adverse events were commonly reported. Total dose amount of sildenafil (mg) taken strongly correlated with % change in nitric oxide metabolite concentration in the plasma (ρ =0.80, p=0.01). A significant increase in plasma and erythrocyte arginine concentration also occurred. Our study suggests that sildenafil is safe and may improve pulmonary hemodynamics in patients at risk for pulmonary hypertension; however efficacy with respect to 6-minute walk distance was not demonstrated. Clinical trials are needed to identify the best treatment strategy for pulmonary hypertension in beta-thalassemia. (clinicaltrials.gov identifier: NCT00872170).
  Haematologica 04/2013;
• Article: Analysis of the immune system of multiple myeloma patients achieving long-term disease control, by multidimensional flow cytometry

  Pessoa-Magalhaes RJ, Vidriales MB, Paiva B, Gimenez CF, Garcia-Sanz R, Mateos MV, Gutierrez N, Lecrevisse Q, Blanco JF, Hernandez J, [......], Roig M, da Costa ES, Ocio E, Perez-Andres M, Maiolino A, Nucci M, Delarubia J, Lahuerta JJ, San Miguel JF, Orfao A
  [show abstract] [hide abstract]
  ABSTRACT: Background. Multiple myeloma remains largely incurable. However, a few patients experience ≥10-years relapse-free survival and can be considered as operationally cured. Interestingly, long-term disease control in multiple myeloma is not restricted to patients with complete response, since some revert into an MGUS profile. Design and Methods. We compared the distribution of multiple compartments of lymphocytes and dendritic cells in the bone marrow and peripheral blood of long-term disease control multiple myeloma (n=28) vs. both newly-diagnosed MGUS (n=23) and symptomatic multiple myeloma (n=23), and age-matched healthy adults (n=10). Results. Similarly to MGUS and symptomatic multiple myeloma, patients with long-term disease control showed an expansion of cytotoxic CD8+T-cells and NK-cells. However, bone marrow Tregs were reduced in long-term disease control vs. symptomatic multiple myeloma. Noteworthy, B-cells were depleted in MGUS and symptomatic multiple myeloma, while recovered in long-term disease control patients in both bone marrow and peripheral blood, due to an increase in normal bone marrow B-cell precursors and plasma cells, as well as pre-germinal center peripheral blood B-cells. The number of bone marrow dendritic cells and tissue macrophages significantly differed between long-term disease control and symptomatic multiple myeloma with a recovery trend in the former patient population towards levels similar to those found in healthy adults.Conclusions. In summary, our results indicate that long-term disease control multiple myeloma patients have a constellation of unique immune changes favoring both immune cytotoxicity and recovery of B-cell production and homing, suggesting an improved immunesurveillance.Keywords: myeloma, long term follow-up, immunesurveillance, T-cells, Tregs, B-cells.
  Haematologica 07/2012; [Epub ahead of print].
• Article: Notch-3 and Notch-4 Signaling promotes the resistance to steroids of Human B-ALL cells in presence of Mesenchymal Stromal Cells

  Nwabo Kamdje AH, Mosna F, Bifari F, Lisi V, Bassi G, Malpeli G, Ricciardi M, Perbellini O, Scupoli MT, Pizzolo G, Krampera M
  Haematologica 10/2011; 96(suppl. 3):1-287.
• Article: Phenotypic and functional evaluation of CD3+CD4-CD8- T cells in human CD8 immunodeficiency. Bernardo I, Mancebo E, Aguiló I, Anel A, Allende LM, Guerra-Vales JM, Ruiz-Contreras J, Serrano A, Talayero P, de la Calle O, Gonzalez-Santesteban C, Paz-Artal E. Haematologica. 2011 Aug;96(8):1195-203.

  Bernardo I, Mancebo E, Aguiló I, Anel A, Allende LM, Guerra-Vales JM, Ruiz-Contreras J, Serrano A, Talayero P, de la Calle O, Gonzalez-Santesteban C, Paz-Artal E
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  ABSTRACT: Abstract BACKGROUND: Human CD8 immunodeficiency is characterized by undetectable CD8(+) lymphocytes and an increased population of CD4(-)CD8(-) (double negative) T lymphocytes. DESIGN AND METHODS: We hypothesized that the double negative subset corresponds to the cellular population that should express CD8 and is committed to the cytotoxic T lymphocyte lineage. To assess this, we determined the phenotype and function of peripheral blood mononuclear cells and/or magnetically isolated double negative T lymphocytes from two CD8-deficient patients. To analyze the expression and co-localization with different organelles, 293T cells were transfected with plasmids bearing wild-type or mutated CD8α. RESULTS: CD8α mutated protein was retained in the cytoplasm of transfected cells. The percentages of double negative cells in patients were lower than the percentages of CD8(+) T cells in healthy controls. Double negative cells mostly had an effector or effector memory phenotype whereas naïve T cells were under-represented. A low concentration of T-cell receptor excision circles together with a skewed T-cell receptor-V repertoire were observed in the double negative population. These data suggest that, in the absence of CD8 co-receptor, the thymic positive selection functions suboptimally and a limited number of mature T-cell clones would emerge from the thymus. In vitro, the double negative cells showed a mild defect in cytotoxic function and decreased proliferative capacity. CONCLUSIONS: It is possible that the double negative cells are major histocompatibility complex class-I restricted T cells with cytolytic function. These results show for the first time in humans that the presence of the CD8 co-receptor is dispensable for cytotoxic ability, but that it affects the generation of thymic precursors committed to the cytotoxic T lymphocyte lineage and the proliferation of mature cytotoxic T cells.
  Haematologica 08/2011; 96(8):1195-203.
• Article: Toll-like receptor signaling pathway in chronic lymphocytic leukemia: Distinct gene expression profiles of potential pathogenetic significance in specific subsets of patients.

  . Arvaniti E., Ntoufa S., Papakonstantinou N., Touloumenidou T., Laoutaris N., Anagnostopoulos A, Lamnissou K, Caligaris-Cappio F, Stamatopoulos K., Ghia P, Muzio M, Belessi C.
  Haematologica 01/2011; 96:1644-1652.
• Article: 279. F. Baron, S. Suciu, S. Amadori, P. Muus, H. Zwierzina, C. Denzlinger, M. Delforge, A. Thyss, D. Selleslag, K. Indrak, G. Ossenkoppele, T. de Witte: Value of infliximab (Remicade®) in patients with low-risk myelodysplastic syndrome: final results of a randomized phase II trial (EORTC trial 06023) of the EORTC Leukemia Group. Haematologica

  F. Baron, S. Suciu, S. Amadori, P. Muus, H. Zwierzina, C. Denzlinger, M. Delforge, A. Thyss, D. Selleslag, K. Indrak, G. Ossenkoppele, T. de Witte
  Haematologica 01/2011;
• Article: HLA-G can be acquired by T cells via trogocytosis and is a clinically relevant immune regulator in patients with myeloma.

  Kabani K, Brown R, Yang S, Aklilu E, Woodland N, Nassif N, Ho P. J, Gibson J. and Joshua D
  Haematologica 01/2011; 96(s1):S37.
• Article: Epidemiological Data Suggest that a Single Biological Event Transforms Myelodysplastic Syndrome to Secondary Acute Myeloid Leukemia

  Ofir Shukron, Vladimir Vainstein, Andrea Kündgen, Ulrich Germing, Zvia Agur
  Haematologica 01/2011;
• Article: THE INTRODUCTION OF SERUM FREE LIGHT CHAINS ASSAY ON THE DIAGNOSIS AND SCREENING PROTOCOLS FOR MONOCLONAL GAMMOPATHIES IS ESSENTIAL TO AN EARLY DIAGNOSIS

  J. Jimenez Jimenez, N. Barbosa de Carvalho, C. Hemando de Larramendi
  Haematologica 01/2010; 95:574.
• Article: Lack of PHLPP1 expression in CLL B cells leads to enhanced AKT activation in response to B-cell receptor stimulation

  M. Suljagic, M. Tarnani, M. Alam, S.N. Malek, L. Laurenti, D.G. Efremov
  Haematologica 10/2009; 94(s3).
• Article: The SYK inhibitor R788 (FosD) inhibits tumor growth in the TCL1 transgenic mouse model of CLL by blocking antigen-dependent BCR signaling

  M. Suljagic, P.G. Longo, L. Laurenti, D.G. Efremov
  Haematologica 10/2009; 94(s3).
• Article: UNUSUAL CAUSE OF DIARRHOEA AFTER UNRELATED HAEMATOPOIETIC STEM CELL TRANSPLANTATION FOR RELAPSED FOLLICULAR LYMPHOMA

  E Sebastian Perez, J Alonso Rodriguez, L Lopez Corral, T Gonzalez, T Caballero Velazquez, J Martin Sanchez, L Vazquez, J San Miguel, T F Flores, A Rodriguez, A Blanco, I Fuentes, G Mcdonald, D Caballero Barrigon
  Haematologica 06/2009; 94}, Meeting Abstract = {1819:688-689.
• Article: ANAPLASTIC LYMPHOMA IN LARGE CELLS IN PEDIATRIC PATIENTS: INTERIM RESULTS OF AIEOP ALCL99 PROTOCOL

  M Pillon, M P Boaro, L Mussolin, G Tridello, A Garaventa, M Nardi, E D'Amore, K Tettoni, A Sala, A Lombardi, R Burnelli, N Santoro, F Spreafico, M Piglione, M L Moleti, G M Fiori, S Varotto, A Rosolen
  Haematologica 10/2008; 93:S27-S28.
• Article: IS THE PLATELET FUNCTION ANALYZER (PFA)-100 A RELIABLE METHOD IN MONITORING ASPIRIN TREATMENT IN PATIENTS WITH THROMBOCYTOSIS?

  E Tsantes, G Mantzios, V Giannopoulou, P Tsirigotis, S Bonovas, E Mygiaki, E Rapti, A Kardoulaki, Z Kartasis, N Sitaras, J Dervenoulas, A Travlou
  Haematologica 06/2008; 93}, Meeting Abstract = {1245:483.
• Article: 205. Faber E., Fiedecký D., Tomková J., Romanová Š., Skoumalová I., Adam T., Jarošová M., Indrák K. (2008): Imatinib plasma levels correlate with molecular response in CML patients. Haematologica: the hematology Journal 93 (1) 219-220.

  Faber E, Fiedecký D, Tomková J, Romanová Š, Skoumalová I, Adam T, Jarošová M, Indrák K
  Haematologica 01/2008;
• Article: 210. Faber E., Friedecký D., Míčová D., Adam T., Indrák K. (2008): Determination of imatinib in plasma by high performance capillary electrophoresis. Haematologica: the hematology Journal 93 (1) 442-443.

  Faber E, Friedecký D, Míčová D, Adam T, Indrák K
  Haematologica 01/2008;
• Article: petlickovski

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  Haematologica 11/2005; 90(11):1533-40.
• Article: Clinical pharmacology of the monoclonal antibodies rituximab and campath-1H

  Iacona I Regazzi MB, Montagna M
  Haematologica 11/2003;